mortality/aging
• all mice die before P12
|
growth/size/body
• severe cardiac enlargement starting from P5
|
• heart weight to body weight and heart weight to tibial length ratios are increased starting at P5
|
• mice exhibit decreased body weight starting at 5 days after birth
|
cardiovascular system
• severe cardiac enlargement starting from P5
|
• heart weight to body weight and heart weight to tibial length ratios are increased starting at P5
|
• severe left ventricle dilation starting from P5 that progresses to thinning of cardiac walls at P10
|
• mice develop severe progressive dilated cardiomyopathy
|
• fractional shortening percentage is reduced by 20% at P1
• however, no differences in fractional shortening percentage are seen at E18.5
|
• echocardiography shows increased left ventricular diameter and reduced systolic cardiac function, with a decrease in fractional shortening percentage
|
homeostasis/metabolism
• collagen positive mural masses are seen in left ventricles from P7 onward, however elastic fibers are absent and fibrin is present in these structures, suggesting they are intracardiac mural thrombi rather than fibroelastosis
|
muscle
• mice develop severe progressive dilated cardiomyopathy
|
• fractional shortening percentage is reduced by 20% at P1
• however, no differences in fractional shortening percentage are seen at E18.5
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
dilated cardiomyopathy 1CC | DOID:0110424 |
OMIM:613122 |
J:290931 |